Cardiac papillary fibroelastomas (CPFs), the second most common primary cardiac tumor, are benign endocardial papillomas predominantly affecting the cardiac valves. Although CPFs are rare and benign tumors, they may result in life-threatening complications. Early diagnosis of this condition is important, since it represents a surgically correctable cause of systemic embolism, myocardial infarction, stroke, acute valve dysfunction, and sudden cardiac death. This review summarizes the significance and clinical approach for the diagnosis of this cardiac entity. The differential diagnosis, histological characteristics of CPF and current treatment strategies are also discussed.